Background: Sickle Cell Anemia (SCA) is an inherited disorder which is associated with significant morbidity and mortality. Early recognition as well as prompt and effective management of the morbidities seen in SCA is key to the survival of patients with the disease. Objective: To determine the morbidity profile and outcome of patients with sickle cell anemia (SCA) admitted into an Emergency Pediatric Unit (EPU) of a tertiary health center in Nigeria. Methods: A retrospective study in which records of admissions of children with SCA over a two-year period (June 2011 to May 2013) were obtained from the EPU records. Information retrieved included age, sex, diagnosis and outcome as far as the unit is concerned. Results: One hundred and forty-seven children, 11.9% of the total (1,234 patients) EPU admission, aged between six months and 18 years (mean age being 84.76 ± 57.24 months) were studied. Male: female ratio was 1.3: 1. Vaso-occlusive (53.2%), hyper-hemolytic (37.6%) and acute sequestration crises (9.2%) were noted but no case of aplastic crisis was seen. Among infections, Malaria (56.2%), Sepsis (15.6%) and pneumonia (12.5%) were the major cases documented. Two (50-0%) patients died of acute sequestration crisis, one (25.0%) of meningitis and another (25.0%) of hyper-hemolytic crisis with severe anemia. Mortality rate was 2.7%. Conclusion/ Recommendation: SCA admissions in our EPU are mainly due to vaso-occlusive crises and infections. Acute sequestration crisis was the leading cause of death followed by meningitis and hyper-hemolytic crisis. Prompt and effective transfusion services, the use of pneumococcal conjugate vaccine as well as judicious use of appropriate antibiotics will augment the chances of survival in these children.
Read full abstract